2004 Letter

“I wish I had cystic fibrosis!”, cried out one of my older daughters at dinner recently. That’s not exactly a question that you’d find in “Parenting 101” so I took a deep breath as I thought of a way out of this one. When asked why she would have such a wish her answer was that Deirdre gets to eat salty, fatty snacks and ice cream; she gets to bounce on the trampoline more than her sisters; and she’s allowed to select which DVD’s we watch.

Sounds like a child’s paradise, huh?

Leah and Theresa know that Deirdre, now 3, has cystic fibrosis (CF) but, of course, they do not know the harsh realty. CF is an inherited genetic disease affecting the glands that produce mucus, tears, sweat, saliva and digestive juices. Normally, these secretions are thin and slippery, but in CF, a defective gene causes the secretions to become thick and sticky. And instead of acting as a lubricant, the secretions may plug up tubes, ducts and passageways, especially in the pancreas and lungs.

Deirdre follows a strict regimen for the treatment of this disease including taking medications such as antibiotics to fight respiratory infections, inhalation therapy, enzymes to aid in her food digestion, and vitamins to improve general health. Children with CF need up to 50% more calories a day so she’s encouraged to supplement her well-balanced diet with additional calories. Also, while Maureen or I perform daily chest physiotherapy on her to dislodge mucus from her lungs, she’s allowed to select the DVD she watches in order to take her mind off of the rhythmic thumping on her chest. After which, she jumps on a trampoline to keep her lung secretions moving.

This is no paradise. While Deirdre is healthy now, her condition will deteriorate based largely upon when, and to what degree, her lungs become affected. Lung infection is inevitable, leading to debility and eventually death. The outlook has improved over the past 25 years, mainly because treatments can now postpone some of the changes that occur in the lungs. Half of the people with CF live longer than 30 years.

Each year the Cystic Fibrosis Foundation sponsors what they call GREAT STRIDES: Taking Steps to Cure Cystic Fibrosis. This is the CF Foundation’s largest national fund-raiser, and it raises money to support innovative research, drug development and care programs dedicated to adding tomorrows every day to the lives of those with CF. More than 90¢ of every dollar raised is invested in research in finding a cure. Last year, with the generosity of our family and friends, we raised nearly $25,000! And, over 35 people joined us in the walk as we proudly wore our “Strides for Deirdre” t-shirts.

Deirdre never complains about her 18 pills a day, the awful tasting vitamins, the daily nebulaizer treatments and chest therapy or the fact that she cannot go visiting one of her cousins if someone in the house has the common cold. She knows no other way… but there is another way. It’s called a cure for CF – now that’s paradise!

Maureen and I greatly appreciate your support and thank you for your time.